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==Overview==
==Overview==
A '''chondrosarcoma''' is a particular [[cancer]] of the [[bone]]. Chondrosarcoma is a cartilage based tumor and is in a category of cancers called [[Sarcoma|sarcomas]]. Chondrosarcoma is a rare cancer that can affect people (and animals) of any age. The aggressiveness of chondrosarcoma is graded based on how fast it grows and its likelihood to metastasize or spread to other parts of the body. Grade 1 is a low grade (slow growing) cancer, and grades 2 and 3 are higher grade (fast growing) cancers. Depending on the grading system used by your medical facility, it's also possible to have grade 4 which would be even more aggressive than lower grade tumors. The most common bones for chondrosarcoma to grow are the pelvic and shoulder bones along with the superior regions of the arms and legs. But they can also be found in any bones of the body, even in the base of the skull.
Chondrosarcoma is the second most common malignant primary tumor of [[bone]]. it is most frequently diagnosed in patients in their 4th and 5th decades of life.Men are slightly more affected with chondrosarcoma than women. There is no racial predilection to chondrosarcoma. Jaffe and Lichtenstein first described chondrosarcoma in 1948. Chondrosarcoma may be classified based on histological findings and location. The exact pathogenesis of chondrosarcoama is not fully understood. Multiple genes have been implicated in pathogenesis of chondrosarcoma. Cytogenetic analysis of chondrosarcomas revealed that structural abnormalities of chromosomes 1, 6, 9, 12 and 15 and numerical abnormalities of chromosomes 5, 7, 8 and 18 are most frequent associated. Anomalies associated with chromosome 9(9p12-22) are more commonly seen in central chondrosarcomas. Germline mutations in the exostosin (EXT1 or EXT2) genes, TP53 or pRb pathway, isocitrate dehydrogenase-1 and isocitrate dehydrogenase 2 genes and gene encoding the receptor for parathyroid have been implicated. On gross pathology, greyish-white lobulated mass, necrosis, calcification, and mucoid degeneration are characteristic findings of chondrosarcoma. On microscopic histopathological analysis abnormal cartilage, increased cellularity, and nuclear atypia are characteristic findings of chondrosarcoma. Chondrosarcoma may be divided into three grades based on cancer cells morphology under microscope and growth rate of tumor. There are no established causes for chondrosarcoma. Common risk factors in the development of chondrosarcoma are benign cartilage tumors such as enchondromas, osteochondromas, multiple exostoses, Ollier's disease, and Maffucci's syndrome. Chondrosarcoma must be differentiated from other diseases such as chondroma, enchondroma, osteochondroma, and osteosarcoma. Complications that can develop as a result of chondrosarcoma are metastasis and recurrence. The prognosis of chondrosarcoma correlates with the grade and stage of the lesion at the time of diagnosis. Chondrosarcoma is associated with a 5 year survival rate of 70%. The presence of grade 3 lesions are associated with a particularly poor prognosis.Biopsy is the gold standard test for the diagnosis of chondrosarcoma. Open biopsy is carried out for chondrosarcoma. The tumor is then staged based on Enneking system for chondrosarcoma. The most common symptoms of chondrosarcoma include pain and swelling in the area of tumor. Patients with chondrosarcoma usually appear lethargic and emaciated. Physical examination of patients with [disease name] is usually remarkable for Palpable masss, tenderness and decreased range of motion. On x-ray, chondrosarcoma is characterized by lytic lesion, intralesional calcification, endosteal scalloping, and cortical remodeling. On CT scan chondrosarcoma is characterized by matrix calcification, endosteal calcification, cortical breach, and heterogenous contrast enhancement. On MRI, chondrosarcoma is characterized by low to intermediate signal on T1, very high intensity in calcified portions on T2, and moderate to intense contrast enhancement on T1 contrast. Bone scan shows very hot uptake in all grades of chondrosarcoma. Chemotherapy and radiotherapy are indicated for chondrosarcoma as adjuvant therapy or palliative treatment in surgically inaccessible areas. Surgery is the mainstay of treatment for chondrosarcoma. Adjunctive chemotherapy and radiation may be required. Recurrence rate depends on the grade of chondrosarcoma.  
 
Nearly all chondrosarcoma patients appear to be in good health. Since it is not like other cancers, it doesn't affect the whole system. Many patients are not aware that there is a tumor growing inside them until there is a noticeable [[lump]] or pain. Sometimes a patient has no symptoms and no awareness of any lump and perhaps, by having a test for something else gets diagnosed accidentally. Sometimes an unexpected [[fracture]] will be the first indication of a [[bone tumor]].
 
==Classification==
Chondrosarcoma may be classified into six subtypes based on topographical location of tumor, histological characteristics, and makeup of surrounding matrix material.
 
==Pathophysiology==
On gross pathology, greyish-white lobulated mass, [[necrosis]], [[calcification]], and mucoid degeneration are characteristic findings of chondrosarcoma. On microscopic histopathological analysis abnormal [[cartilage]], increased cellularity, and nuclear atypia are characteristic findings of chondrosarcoma. Chondrosarcoma may be divided into three grades based on cancer cells morphology under microscope and growth rate of [[tumor]].
 
==Causes==
There are no established causes for chondrosarcoma.
 
==Differential Diagnosis==
Chondrosarcoma must be differentiated from other diseases such as [[chondroma]], [[enchondroma]], [[synovial chondromatosis]], and [[osteosarcoma]].
 
==Epidemiology and Demographics==
Chondrosarcoma is the second most common malignant primary tumor of [[bone]]. Men are slightly more affected with liposarcoma than women. It is most frequent in middle-aged and older adults.
 
==Risk factors==
Common risk factors in the development of chondrosarcoma are benign cartilage tumors such as [[enchondromas]], [[osteochondromas]], multiple exostoses, [[Ollier's disease]], and [[Maffucci's syndrome]].
 
==Natural History, Complications and Prognosis==
Complications that can develop as a result of chondrosarcoma are [[metastasis]] and recurrence. The prognosis of chondrosarcoma is good with treatment. Chondrosarcoma is associated with a 5 year survival rate of 75%. The presence of grade 3 lesions are associated with a particularly poor [[prognosis]].
 
==Diagnosis==
===History and Symptoms===
The most common symptoms of chondrosarcoma include [[pain]] and [[swelling]] in the area of [[tumor]].
===Physical Examination===
Physical examination findings will depend on the location of the chondrosarcoma. Common physical examination findings of chondrosarcoma are localized [[swelling]] and [[tenderness]].
===Laboratory Findings===
There are no specific laboratory tests for the diagnosis of chondrosarcoma.
===X Ray===
On x-ray, chondrosarcoma is characterized by lytic lesion, intralesional [[calcification]], endosteal scalloping, and cortical remodeling.<ref>{{Cite web | title =chondrosarcoma radiograph| url =http://radiopaedia.org/articles/chondrosarcoma }}</ref>
===CT===
On CT scan, chondrosarcoma is characterized by [[Calcification|matrix calcification]], endosteal calcification, cortical breach, and heterogenous contrast enhancement.
 
===MRI===
On MRI, chondrosarcoma is characterized by low to intermediate signal on T1, very high intensity in calcified portions on T2, and moderate to intense contrast enhancement on T1 contrast.
 
==Treatment==
===Medical Therapy===
[[Chemotherapy]] and [[radiotherapy]] are indicated for chondrosarcoma as [[adjuvant therapy]]. [[Proton therapy]] is a new kind of radiotherapy showing promising results with local tumor control at over 80%.<ref>{{Cite web | title =Center for Proton Therapy| url =http://p-therapie.web.psi.ch/e/klinikerfahrung.html }}</ref>
 
===Surgery===
The predominant therapy for chondrosarcoma is surgical resection. Adjunctive [[chemotherapy]] and [[radiation]] may be required.


==References==
==References==
{{reflist|2}}
{{reflist|2}}


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Revision as of 15:02, 24 January 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Rohan A. Bhimani, M.B.B.S., D.N.B., M.Ch.[2]

Overview

Chondrosarcoma is the second most common malignant primary tumor of bone. it is most frequently diagnosed in patients in their 4th and 5th decades of life.Men are slightly more affected with chondrosarcoma than women. There is no racial predilection to chondrosarcoma. Jaffe and Lichtenstein first described chondrosarcoma in 1948. Chondrosarcoma may be classified based on histological findings and location. The exact pathogenesis of chondrosarcoama is not fully understood. Multiple genes have been implicated in pathogenesis of chondrosarcoma. Cytogenetic analysis of chondrosarcomas revealed that structural abnormalities of chromosomes 1, 6, 9, 12 and 15 and numerical abnormalities of chromosomes 5, 7, 8 and 18 are most frequent associated. Anomalies associated with chromosome 9(9p12-22) are more commonly seen in central chondrosarcomas. Germline mutations in the exostosin (EXT1 or EXT2) genes, TP53 or pRb pathway, isocitrate dehydrogenase-1 and isocitrate dehydrogenase 2 genes and gene encoding the receptor for parathyroid have been implicated. On gross pathology, greyish-white lobulated mass, necrosis, calcification, and mucoid degeneration are characteristic findings of chondrosarcoma. On microscopic histopathological analysis abnormal cartilage, increased cellularity, and nuclear atypia are characteristic findings of chondrosarcoma. Chondrosarcoma may be divided into three grades based on cancer cells morphology under microscope and growth rate of tumor. There are no established causes for chondrosarcoma. Common risk factors in the development of chondrosarcoma are benign cartilage tumors such as enchondromas, osteochondromas, multiple exostoses, Ollier's disease, and Maffucci's syndrome. Chondrosarcoma must be differentiated from other diseases such as chondroma, enchondroma, osteochondroma, and osteosarcoma. Complications that can develop as a result of chondrosarcoma are metastasis and recurrence. The prognosis of chondrosarcoma correlates with the grade and stage of the lesion at the time of diagnosis. Chondrosarcoma is associated with a 5 year survival rate of 70%. The presence of grade 3 lesions are associated with a particularly poor prognosis.Biopsy is the gold standard test for the diagnosis of chondrosarcoma. Open biopsy is carried out for chondrosarcoma. The tumor is then staged based on Enneking system for chondrosarcoma. The most common symptoms of chondrosarcoma include pain and swelling in the area of tumor. Patients with chondrosarcoma usually appear lethargic and emaciated. Physical examination of patients with [disease name] is usually remarkable for Palpable masss, tenderness and decreased range of motion. On x-ray, chondrosarcoma is characterized by lytic lesion, intralesional calcification, endosteal scalloping, and cortical remodeling. On CT scan chondrosarcoma is characterized by matrix calcification, endosteal calcification, cortical breach, and heterogenous contrast enhancement. On MRI, chondrosarcoma is characterized by low to intermediate signal on T1, very high intensity in calcified portions on T2, and moderate to intense contrast enhancement on T1 contrast. Bone scan shows very hot uptake in all grades of chondrosarcoma. Chemotherapy and radiotherapy are indicated for chondrosarcoma as adjuvant therapy or palliative treatment in surgically inaccessible areas. Surgery is the mainstay of treatment for chondrosarcoma. Adjunctive chemotherapy and radiation may be required. Recurrence rate depends on the grade of chondrosarcoma.

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